Primary localized nodular tongue amyloidosis associated with Sjögren's syndrome

We describe a case of primary localized nodular tongue amyloidosis associated with Sjögren's syndrome in a 62-year-old woman. The presence of Sjögren's syndrome was confirmed both serologically and histologically. The amyloid tumor, which was marginally excised, recurred 3 years later and was re-excised. Immunohistochemical examination revealed that the amyloid protein was of the AL (lambda-light chain) type. Infiltration of plasma cells was observed around the minor salivary glands of the tongue surrounded by amyloid. The relationship between the plasma cells and amyloid deposition is discussed.